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[PMID]:28847111
[Au] Autor:Sharma S
[Ad] Endereço:Department of Neurosurgery, Trivandrum Medical College, Kerala, India. Electronic address: ashu20030000@gmail.com.
[Ti] Título:Rosette-Forming Glioneuronal Tumor Arising from the Spinal Cord.
[So] Source:World Neurosurg;105:1001, 2017 09.
[Is] ISSN:1878-8769
[Cp] País de publicação:United States
[La] Idioma:eng
[Mh] Termos MeSH primário: Neoplasias Neuroepiteliomatosas
Medula Espinal
[Mh] Termos MeSH secundário: Neoplasias do Ventrículo Cerebral
Quarto Ventrículo
Ganglioglioma
Seres Humanos
[Pt] Tipo de publicação:LETTER; COMMENT
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170914
[Lr] Data última revisão:
170914
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170830
[St] Status:MEDLINE


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[PMID]:28804898
[Au] Autor:Giulioni M; Marucci G; Pelliccia V; Gozzo F; Barba C; Didato G; Villani F; Di Gennaro G; Quarato PP; Esposito V; Consales A; Martinoni M; Vornetti G; Zenesini C; Efisio Marras C; Specchio N; De Palma L; Rocchi R; Giordano F; Tringali G; Nozza P; Colicchio G; Rubboli G; Lo Russo G; Guerrini R; Tinuper P; Cardinale F; Cossu M; Commission for Epilepsy Surgery of the Italian League Against Epilepsy
[Ad] Endereço:Division of Neurosurgery, IRCCS Institute of Neurological Sciences of Bologna, Bologna, Italy.
[Ti] Título:Epilepsy surgery of "low grade epilepsy associated neuroepithelial tumors": A retrospective nationwide Italian study.
[So] Source:Epilepsia;58(11):1832-1841, 2017 Nov.
[Is] ISSN:1528-1167
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:OBJECTIVE: To analyze the attitude and results of Italian epilepsy surgery centers in the surgical management of "low grade epilepsy associated neuroepithelial tumors" (LEATs). METHODS: We conducted a retrospective study enrolling 339 consecutive patients with LEATs who underwent surgery between January 2009 and June 2015 at eight Italian epilepsy surgery centers. We compared demographic, clinical, pathologic, and surgical features of patients with favorable (Engel class I) and unfavorable (Engel class II, III, and IV) seizure outcome. In addition, we compared patients with tumor-associated focal cortical dysplasia (FCD) and patients with solitary tumors to identify factors correlated with FCD diagnosis. RESULTS: Fifty-five (98.2%) of 56 patients with medically controlled epilepsy were seizure-free after surgery, compared to 249 (88.0%) of 283 patients with refractory epilepsy. At multivariate analysis, three variables independently predict unfavorable seizure outcome in the drug-resistant group. Age at surgery is largely the most significant (p = 0.001), with an odds ratio (OR) of 1.04. This means that the probability of seizure recurrence grows by 4% for every waited year. The resection site is also significant (p = 0.039), with a relative risk (RR) of 1.99 for extratemporal tumors. Finally, the completeness of tumor resection has a trend toward significance (p = 0.092), with an RR of 1.82 for incomplete resection. Among pediatric patients, a longer duration of epilepsy was significantly associated with preoperative neuropsychological deficits (p < 0.001). A statistically significant association was observed between FCD diagnosis and the following variables: tailored surgery (p < 0.001), temporal resection (p = 0.001), and surgical center (p = 0.012). SIGNIFICANCE: Our nationwide LEATs study gives important insights on factors predicting seizure outcome in refractory epilepsy and determining variability in FCD detection. Timely surgery, regardless of pharmacoresistance and oriented to optimize epileptologic, neuropsychological, and oncologic outcomes should be warranted.
[Mh] Termos MeSH primário: Neoplasias Encefálicas/epidemiologia
Neoplasias Encefálicas/cirurgia
Epilepsia Resistente a Medicamentos/epidemiologia
Epilepsia Resistente a Medicamentos/cirurgia
Neoplasias Neuroepiteliomatosas/epidemiologia
Neoplasias Neuroepiteliomatosas/cirurgia
[Mh] Termos MeSH secundário: Adolescente
Adulto
Neoplasias Encefálicas/diagnóstico
Criança
Epilepsia Resistente a Medicamentos/diagnóstico
Feminino
Seguimentos
Seres Humanos
Itália/epidemiologia
Masculino
Neoplasias Neuroepiteliomatosas/diagnóstico
Estudos Retrospectivos
Resultado do Tratamento
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE; MULTICENTER STUDY
[Em] Mês de entrada:1711
[Cu] Atualização por classe:171109
[Lr] Data última revisão:
171109
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170815
[St] Status:MEDLINE
[do] DOI:10.1111/epi.13866


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[PMID]:28704208
[Au] Autor:Appay R; Macagno N; Padovani L; Korshunov A; Kool M; André N; Scavarda D; Pietsch T; Figarella-Branger D
[Ad] Endereço:Departments of *Pathology and Neuropathology †Radiotherapy ¶Hematology & Pediatric Oncology **Pediatric Neurosurgery, Timone Hospital, France #INSERM, CRO2 UMR_S 911, Aix-Marseille Université (AMU) ‡Clinical Cooperation Unit Neuropathology §Division of Pediatric Neurooncology, German Cancer Research Center (DKFZ) ∥Cancer Consortium (DKTK), Core Center Heidelberg, Heidelberg ††Department of Neuropathology, Medical Center Sigmund Freud, University of Bonn, Bonn, Germany.
[Ti] Título:HGNET-BCOR Tumors of the Cerebellum: Clinicopathologic and Molecular Characterization of 3 Cases.
[So] Source:Am J Surg Pathol;41(9):1254-1260, 2017 Sep.
[Is] ISSN:1532-0979
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:The central nervous system (CNS) high-grade neuroepithelial tumor with BCOR alteration (CNS HGNET-BCOR) is a recently described molecular entity. We report 3 new CNS HGNET-BCOR cases sharing common clinical presentation and pathologic features. The 3 cases concerned children aged 3 to 7 years who presented with a voluminous mass of the cerebellum. Pathologic features included proliferation of uniform spindle to ovoid cells with fine chromatin associated with a rich arborizing capillary network. Methylation profiling classified these cases as CNS HGNET-BCOR tumors. Polymerase chain reaction analysis confirmed the presence of internal tandem duplications in the C-terminus of BCOR (BCOR-ITD), a characteristic of these tumors, in all 3 cases. Immunohistochemistry showed a strong nuclear BCOR expression. In 2 cases, local recurrence occurred within 6 months. The third case, a patient who received a craniospinal irradiation after total surgical removal followed by a metronomics maintenance with irinotecan, temozolomide, and itraconazole, is still free of disease 14 months after diagnosis. In summary, CNS HGNET-BCOR represents a rare tumor occurring in young patients with dismal prognosis. BCOR nuclear immunoreactivity is highly suggestive of a BCOR-ITD. Whether CNS HGNET-BCOR should be classified among the category of "embryonal tumors" or within the category of "mesenchymal, nonmeningothelial tumors" remains to be clarified. Because CNS HGNET-BCOR share pathologic features and characteristic BCOR-ITD with clear cell sarcoma of the kidney, these tumors may represent local variants of the same entity.
[Mh] Termos MeSH primário: Neoplasias Cerebelares/genética
Neoplasias Cerebelares/patologia
Neoplasias Neuroepiteliomatosas/genética
Neoplasias Neuroepiteliomatosas/patologia
Proteínas Proto-Oncogênicas/genética
Proteínas Repressoras/genética
[Mh] Termos MeSH secundário: Criança
Pré-Escolar
Feminino
Seres Humanos
Masculino
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (BCOR protein, human); 0 (Proto-Oncogene Proteins); 0 (Repressor Proteins)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170914
[Lr] Data última revisão:
170914
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170714
[St] Status:MEDLINE
[do] DOI:10.1097/PAS.0000000000000866


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[PMID]:28409885
[Au] Autor:El Madi A; Irtan S; Sauvat F; Zérah M; Schleiermacher G; Galmiche-Roland L; Minard-Colin V; Brisse H; Sarnacki S
[Ad] Endereço:Department of Pediatric Surgery, Fez University Hospital, Fez, Morocco.
[Ti] Título:Long-term results of the transmanubrial osteomuscular-sparing approach for pediatric tumors.
[So] Source:Pediatr Blood Cancer;64(10), 2017 Oct.
[Is] ISSN:1545-5017
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: The complete and safe resection of pediatric cervicothoracic tumors, mostly represented by neurogenic tumors, remains a surgical challenge because of the complex anatomy of this region. The transmanubrial osteomuscular-sparing approach (TOSA) is an alternative to isolated or combined cervical and thoracic approaches enabling the control of supra-aortic vessels and nerves through the thoracic inlet. METHODS: We retrospectively reviewed the tumor characteristics, completeness of resection, morbidity, and long-term outcome of patients with cervicothoracic tumors removed by TOSA between 2000 and 2012 in our institution. RESULTS: Thirteen patients (7 males, 6 females) underwent surgery at a median age of 72 months (4-188) for neuroblastoma (n = 6), ganglioneuroblastoma (n = 3), rhabdoid tumor (n = 1), melanotic schwannoma (n = 1), chordoma (n = 1), and malignant peripheral nerve sheath tumor in one patient with type 1 neurofibromatosis. The median duration of the procedure was 215 minutes (110-315). Two children presented with postoperative chylothorax that resolved spontaneously. The median duration of hospitalization was 7 days (4-22). At a median follow-up of 39 months (2-159), four patients had died of metastatic relapse (n = 2), locoregional progression (n = 1), and chemotoxicity (n = 1). The patient with melanotic schwannoma was lost to follow-up after a local relapse at 5 months. Long-term morbidity revealed homolateral Claude-Bernard Horner sign and upper limb vasomotor dysfunction in disease-free patients due to mandatory resection of the stellate ganglia. CONCLUSIONS: TOSA is a valuable surgical approach for all cervicothoracic tumors with acceptable long-term morbidity when compared with its complexity. We can therefore recommend TOSA for tumors involving the thoracic inlet.
[Mh] Termos MeSH primário: Neoplasias de Cabeça e Pescoço
Neoplasias Neuroepiteliomatosas
Neoplasias Torácicas
[Mh] Termos MeSH secundário: Adolescente
Criança
Pré-Escolar
Feminino
Seguimentos
Neoplasias de Cabeça e Pescoço/mortalidade
Neoplasias de Cabeça e Pescoço/patologia
Neoplasias de Cabeça e Pescoço/cirurgia
Seres Humanos
Lactente
Masculino
Neoplasias Neuroepiteliomatosas/mortalidade
Neoplasias Neuroepiteliomatosas/patologia
Neoplasias Neuroepiteliomatosas/cirurgia
Estudos Retrospectivos
Neoplasias Torácicas/mortalidade
Neoplasias Torácicas/patologia
Neoplasias Torácicas/cirurgia
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171016
[Lr] Data última revisão:
171016
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170415
[St] Status:MEDLINE
[do] DOI:10.1002/pbc.26527


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[PMID]:28408259
[Au] Autor:Fu W; Ju Y; Zhang S; You C
[Ad] Endereço:Department of Neurosurgery, West China Hospital of Sichuan University, Chengdu, Sichuan, China.
[Ti] Título:Pediatric Basal Ganglia Region Tumors: Clinical and Radiologic Features Correlated with Histopathologic Findings.
[So] Source:World Neurosurg;103:504-516, 2017 Jul.
[Is] ISSN:1878-8769
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:PURPOSE: To summarize the clinical and radiologic features of pediatric basal ganglia region tumors (PBGRT) in correlation with their histopathologic findings to reduce inappropriate surgery and identify tumors that can benefit from maximal safe resection. METHODS: The records of 35 children with PBGRT treated in our hospital from December 2011 to December 2015 were analyzed retrospectively. The clinical and radiologic features of these tumors were summarized and correlated with their histopathologic diagnosis. RESULTS: Our series included 15 astrocytomas and 11 germ cell tumors (GCTs). Basal ganglia astrocytomas were characterized by various clinical presentations and an ill-circumscribed mass with the involvement of surrounding structures on neuroimaging and mostly occurred in the first decade of life (n = 10; 66.7%). Basal ganglia GCT mostly occurred in the second decade of life (n = 8; 72.7%) with hemiparesis as the most common symptom (n = 9; 81.8%). The tumors were located predominantly in the caput of caudate nucleus (n = 8; 72.7%) with hemiatrophy as the typical sign (n = 8; 72.7%). Occasionally, other tumors also could occur in this region, including primitive neuroectodermal tumor (n = 1), atypical teratoid/rhabdoid tumor (n = 1), anaplastic ependymoma (n = 1), lymphoma (n = 1), extraventricular neurocytoma (n = 1), gangliogliomas (n = 2), oligodendroglioma (n = 1), and dysembryoplastic neuroepithelial tumor (n = 1). CONCLUSIONS: Astrocytoma and GCT are the most common PBGRTs. Low-grade astrocytomas could benefit from maximal surgical resection, whereas GCTs merit neoadjuvant chemoradiation therapy followed by second-look surgery. We advocate routine testing of tumor markers and analysis of their clinical and radiologic features to optimize the therapeutic strategy.
[Mh] Termos MeSH primário: Astrocitoma/terapia
Doenças dos Gânglios da Base/terapia
Biomarcadores Tumorais/metabolismo
Neoplasias Encefálicas/terapia
Quimiorradioterapia
Terapia Neoadjuvante
Neoplasias Embrionárias de Células Germinativas/terapia
Procedimentos Neurocirúrgicos
[Mh] Termos MeSH secundário: Adolescente
Astrocitoma/complicações
Astrocitoma/diagnóstico por imagem
Astrocitoma/metabolismo
Doenças dos Gânglios da Base/complicações
Doenças dos Gânglios da Base/diagnóstico por imagem
Doenças dos Gânglios da Base/metabolismo
Neoplasias Encefálicas/complicações
Neoplasias Encefálicas/diagnóstico por imagem
Neoplasias Encefálicas/metabolismo
Núcleo Caudado/diagnóstico por imagem
Núcleo Caudado/cirurgia
Criança
Pré-Escolar
Tontura/etiologia
Ependimoma/complicações
Ependimoma/diagnóstico por imagem
Ependimoma/metabolismo
Ependimoma/terapia
Feminino
Ganglioglioma/complicações
Ganglioglioma/diagnóstico por imagem
Ganglioglioma/metabolismo
Ganglioglioma/terapia
Cefaleia/etiologia
Seres Humanos
Lactente
Linfoma/complicações
Linfoma/diagnóstico por imagem
Linfoma/metabolismo
Linfoma/terapia
Masculino
Técnicas de Diagnóstico Molecular
Neoplasias Embrionárias de Células Germinativas/complicações
Neoplasias Embrionárias de Células Germinativas/diagnóstico por imagem
Neoplasias Embrionárias de Células Germinativas/metabolismo
Neoplasias Neuroepiteliomatosas/complicações
Neoplasias Neuroepiteliomatosas/diagnóstico por imagem
Neoplasias Neuroepiteliomatosas/terapia
Neurocitoma/complicações
Neurocitoma/diagnóstico por imagem
Neurocitoma/metabolismo
Neurocitoma/terapia
Tumores Neuroectodérmicos Primitivos/complicações
Tumores Neuroectodérmicos Primitivos/diagnóstico por imagem
Tumores Neuroectodérmicos Primitivos/metabolismo
Tumores Neuroectodérmicos Primitivos/terapia
Oligodendroglioma/complicações
Oligodendroglioma/diagnóstico por imagem
Oligodendroglioma/metabolismo
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Biomarkers, Tumor)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170925
[Lr] Data última revisão:
170925
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170415
[St] Status:MEDLINE


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[PMID]:28369089
[Au] Autor:Werneck de Carvalho LE; Sarraf JS; Semblano AA; Moreira MA; de Lemos MN; de Mello VJ; Hamoy M; Nazareth Junior MH; Paschoal Junior FM; Adami F
[Ad] Endereço:Oncológica Brasil-Ensino e Pesquisa, Belém, Pará, Brazil.
[Ti] Título:Central nervous system tumours profile at a referral center in the Brazilian Amazon region, 1997-2014.
[So] Source:PLoS One;12(4):e0174439, 2017.
[Is] ISSN:1932-6203
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Tumours of the Central Nervous System (CNS) are an important cause of mortality from cancer. Epidemiological data on neoplams affecting the CNS are scarce in Brazil, especially in the Amazon region. The study aims at describing the histopathological profile of CNS tumours cases at a high-complexity referral cancer center. This study has described a 17-year-series profile of CNS tumours, registered at a high-complexity referral cancer center in Pará state, from January 1997 until July 2014 in the Brazilian Amazon Region. Data was gathered from histopathology reports kept in the hospital's cancer registry and 949 cases of CNS tumours were analyzed. The most common histopathology were neuroepithelial tumours (approx. 40%) and meningioma was the most frequent especific tumor histologic subtype (22.2%). Neuroepithelial tumours were more frequent in patients with ages ranging from less than a year to 19 years, whereas metastatic tumours were prevalent in patients over 40 years of age. It was not found temporal trends during the studied period. The knowledge of these tumours profile is valuable for the understanding of cancer epidemiology in the region, since its prevalence is currently underreported and more awareness on the disease is needed.
[Mh] Termos MeSH primário: Neoplasias do Sistema Nervoso Central/epidemiologia
[Mh] Termos MeSH secundário: Adolescente
Adulto
Idoso
Idoso de 80 Anos ou mais
Brasil/epidemiologia
Neoplasias do Sistema Nervoso Central/patologia
Neoplasias do Sistema Nervoso Central/secundário
Criança
Pré-Escolar
Feminino
Seres Humanos
Lactente
Recém-Nascido
Masculino
Meia-Idade
Neoplasias Neuroepiteliomatosas/epidemiologia
Neoplasias Neuroepiteliomatosas/patologia
Prevalência
Encaminhamento e Consulta
Sistema de Registros
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170906
[Lr] Data última revisão:
170906
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170404
[St] Status:MEDLINE
[do] DOI:10.1371/journal.pone.0174439


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[PMID]:28366748
[Au] Autor:Carroll KT; Hirshman B; Ali MA; Alattar AA; Brandel MG; Lochte B; Lanman T; Carter B; Chen CC
[Ad] Endereço:School of Medicine, University of California, San Diego, La Jolla, California, USA.
[Ti] Título:Management and Survival Patterns of Patients with Gliomatosis Cerebri: A SEER-Based Analysis.
[So] Source:World Neurosurg;103:186-193, 2017 Jul.
[Is] ISSN:1878-8769
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:OBJECTIVE: We used the SEER (Surveillance Epidemiology and End Results) database (1999-2010) to analyze the clinical practice patterns and overall survival in patients with gliomatosis cerebri (GC), or glioma involving 3 or more lobes of the cerebrum. METHODS: We identified 111 patients (age ≥18 years) with clinically or microscopically diagnosed GC in the SEER database. Analyses were performed to determine clinical practice patterns for these patients and whether these practices were associated with survival. RESULTS: Fifty-eight percent of the 111 patients with GC received microscopic confirmation of their diagnosis. Of the remaining patients, 40% were diagnosed via imaging or laboratory tests, and 2% had unknown methods of diagnosis. Seven percent of patients who did not have microscopic confirmation of their diagnosis received radiation therapy. Radiation therapy and surgery were not associated with survival. The only variable significantly associated with overall survival was age at diagnosis. Patients aged 18-50 years showed improved survival relative to patients aged >50 years (median survival, 11 and 6 months, respectively; P = 0.03). For patients aged >50 years, improved overall survival was observed in the post-temozolomide era (2005-2010) relative to those treated in the pre-temozolomide era (1999-2004) (median survival, 9 and 4 months, respectively; P = 0.005). CONCLUSIONS: In the SEER database, ∼40% of the patients with glioma with imaging findings of GC do not receive microscopic confirmation of their diagnosis. We propose that tissue confirmation is warranted in patients with GC, because genomic analysis of these specimens may provide insights that will contribute to meaningful therapeutic intervention.
[Mh] Termos MeSH primário: Antineoplásicos Alquilantes/uso terapêutico
Astrocitoma/terapia
Neoplasias Encefálicas/terapia
Dacarbazina/análogos & derivados
Glioblastoma/terapia
Neoplasias Primárias Múltiplas/terapia
Oligodendroglioma/terapia
[Mh] Termos MeSH secundário: Adulto
Fatores Etários
Idoso
Idoso de 80 Anos ou mais
Astrocitoma/mortalidade
Neoplasias Encefálicas/mortalidade
Terapia Combinada
Irradiação Craniana
Dacarbazina/uso terapêutico
Gerenciamento Clínico
Feminino
Glioblastoma/mortalidade
Glioma/mortalidade
Glioma/terapia
Seres Humanos
Estimativa de Kaplan-Meier
Masculino
Meia-Idade
Neoplasias Primárias Múltiplas/mortalidade
Neoplasias Neuroepiteliomatosas/mortalidade
Neoplasias Neuroepiteliomatosas/terapia
Procedimentos Neurocirúrgicos
Oligodendroglioma/mortalidade
Modelos de Riscos Proporcionais
Estudos Retrospectivos
Programa de SEER
Taxa de Sobrevida
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Antineoplastic Agents, Alkylating); 7GR28W0FJI (Dacarbazine); YF1K15M17Y (temozolomide)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170925
[Lr] Data última revisão:
170925
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170404
[St] Status:MEDLINE


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[PMID]:28328117
[Au] Autor:Siegfried A; Cances C; Denuelle M; Loukh N; Tauber M; Cavé H; Delisle MB
[Ad] Endereço:Department of Pathology, Institut Universitaire du Cancer, Oncopole, Toulouse, France.
[Ti] Título:Noonan syndrome, PTPN11 mutations, and brain tumors. A clinical report and review of the literature.
[So] Source:Am J Med Genet A;173(4):1061-1065, 2017 Apr.
[Is] ISSN:1552-4833
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Noonan syndrome (NS), an autosomal dominant disorder, is characterized by short stature, congenital heart defects, developmental delay, and facial dysmorphism. PTPN11 mutations are the most common cause of NS. PTPN11 encodes a non-receptor protein tyrosine phosphatase, SHP2. Hematopoietic malignancies and solid tumors are associated with NS. Among solid tumors, brain tumors have been described in children and young adults but remain rather rare. We report a 16-year-old boy with PTPN11-related NS who, at the age of 12, was incidentally found to have a left temporal lobe brain tumor and a cystic lesion in the right thalamus. He developed epilepsy 2 years later. The temporal tumor was surgically resected because of increasing crises and worsening radiological signs. Microscopy showed nodules with specific glioneuronal elements or glial nodules, leading to the diagnosis of dysembryoplastic neuroepithelial tumor (DNT). Immunohistochemistry revealed positive nuclear staining with Olig2 and pERK in small cells. SHP2 plays a key role in RAS/MAPK pathway signaling which controls several developmental cell processes and oncogenesis. An amino-acid substitution in the N-terminal SHP2 domain disrupts the self-locking conformation and leads to ERK activation. Glioneuronal tumors including DNTs and pilocytic astrocytomas have been described in NS. This report provides further support for the relation of DNTs with RASopathies and for the implication of RAS/MAPK pathways in sporadic low-grade glial tumors including DNTs. © 2017 Wiley Periodicals, Inc.
[Mh] Termos MeSH primário: Neoplasias Encefálicas/genética
Epilepsia/genética
Mutação
Neoplasias Neuroepiteliomatosas/genética
Síndrome de Noonan/genética
Proteína Tirosina Fosfatase não Receptora Tipo 11/genética
[Mh] Termos MeSH secundário: Adolescente
Adulto
Fatores de Transcrição Hélice-Alça-Hélice Básicos/genética
Fatores de Transcrição Hélice-Alça-Hélice Básicos/metabolismo
Neoplasias Encefálicas/diagnóstico
Neoplasias Encefálicas/patologia
Neoplasias Encefálicas/cirurgia
Criança
Epilepsia/diagnóstico
Epilepsia/patologia
Epilepsia/cirurgia
MAP Quinases Reguladas por Sinal Extracelular/genética
MAP Quinases Reguladas por Sinal Extracelular/metabolismo
Expressão Gênica
Genes Dominantes
Seres Humanos
Masculino
Neoplasias Neuroepiteliomatosas/diagnóstico
Neoplasias Neuroepiteliomatosas/patologia
Neoplasias Neuroepiteliomatosas/cirurgia
Proteínas do Tecido Nervoso/genética
Proteínas do Tecido Nervoso/metabolismo
Síndrome de Noonan/diagnóstico
Síndrome de Noonan/patologia
Síndrome de Noonan/cirurgia
Fator de Transcrição 2 de Oligodendrócitos
Proteína Tirosina Fosfatase não Receptora Tipo 11/metabolismo
Lobo Temporal/metabolismo
Lobo Temporal/patologia
Lobo Temporal/cirurgia
Tálamo/metabolismo
Tálamo/patologia
Tálamo/cirurgia
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE; REVIEW
[Nm] Nome de substância:
0 (Basic Helix-Loop-Helix Transcription Factors); 0 (Nerve Tissue Proteins); 0 (OLIG2 protein, human); 0 (Oligodendrocyte Transcription Factor 2); EC 2.7.11.24 (Extracellular Signal-Regulated MAP Kinases); EC 3.1.3.48 (PTPN11 protein, human); EC 3.1.3.48 (Protein Tyrosine Phosphatase, Non-Receptor Type 11)
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171116
[Lr] Data última revisão:
171116
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170323
[St] Status:MEDLINE
[do] DOI:10.1002/ajmg.a.38108


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[PMID]:28291213
[Au] Autor:Kim AV; Khachatryan VA
[Ad] Endereço:Polenov Neurosurgical Institute, Branch of the Almazov Federal North-West Medical Research Centre, Saint-Petersburg, Russia.
[Ti] Título:[Intraoperative fluorescence diagnosis using 5-aminolevulinic acid in surgical treatment of children with recurrent neuroepithelial tumors].
[Ti] Título:Rezul'taty intraoperatsionnoi fluorestsentnoi diagnostiki s ispol'zovaniem 5-aminolevulinovoi kisloty pri khirurgicheskom lechenii detei s retsidivami neiroepitelial'nykh opukholei..
[So] Source:Zh Vopr Neirokhir Im N N Burdenko;81(1):51-57, 2017.
[Is] ISSN:0042-8817
[Cp] País de publicação:Russia (Federation)
[La] Idioma:rus
[Ab] Resumo:AIM: To analyze the efficacy and safety of 5-aminolevulinic acid for intraoperative fluorescence diagnosis (FD) during repeated surgery for recurrent neuroepithelial tumors (NETs) in children. MATERIAL AND METHODS: We performed a retrospective analysis of the results of recurrent NET resection in 13 children aged from 3 to 17 years in the period between December 2013 and May 2015. The source of 5-ALA was a drug "Alasens" that was administered at a dose of 20 mg/kg of body weight 4 h before the beginning of tumor resection. A visual scale was used to evaluate the fluorescence intensity. The drug was used with the informed consent of the child's parents and approval of the Polenov Neurosurgical Institute ethics committee. All patients underwent complex clinical and introscopic examination in the pre- and postoperative periods and a repeated histological tumor examination. RESULTS: There was no fluorescence in 4 cases (Gr I in 2 cases; Gr II in 2 cases). Grade 1 fluorescence (Gr II) was observed in one case, and grade 2-3 fluorescence was observed in the remaining 8 patients. Total and subtotal tumor resection was achieved in 11 (84.6%) of 13 patients. There were no clinically significant adverse effects. A transient increase in transaminases was observed in 2 patients. Two girls had an elevated photosensitivity to light for two days. CONCLUSION: FD is a safe technique in childhood and can improve intraoperative detection of NET during its repeated growth as well as differentiate the tumor from pathological tissues of non-tumorous nature, which increases completeness of blastomatous tissue removal. However, the character and intensity of fluorescence may change in recurrent NETs. Further research is required to investigate the FD effectiveness in various age patients with tumors of a different histological structure and malignancy in the case of both primary and recurrent lesions.
[Mh] Termos MeSH primário: Ácido Aminolevulínico/administração & dosagem
Neoplasias Encefálicas
Cuidados Intraoperatórios/métodos
Neoplasias Neuroepiteliomatosas
Imagem Óptica/métodos
[Mh] Termos MeSH secundário: Adolescente
Neoplasias Encefálicas/diagnóstico por imagem
Neoplasias Encefálicas/cirurgia
Criança
Pré-Escolar
Feminino
Seres Humanos
Masculino
Neoplasias Neuroepiteliomatosas/diagnóstico por imagem
Neoplasias Neuroepiteliomatosas/cirurgia
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
88755TAZ87 (Aminolevulinic Acid)
[Em] Mês de entrada:1706
[Cu] Atualização por classe:170614
[Lr] Data última revisão:
170614
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170315
[St] Status:MEDLINE
[do] DOI:10.17116/neiro201780751-57


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[PMID]:28232116
[Au] Autor:Coban EA; Kasikci E; Karatas OF; Suakar O; Kuskucu A; Altunbek M; Türe U; Sahin F; Bayrak OF
[Ad] Endereço:Department of Genetics and Bioengineering, Faculty of Engineering and Architecture, Yeditepe University, Istanbul, Turkey.
[Ti] Título:Characterization of stem-like cells in a new astroblastoma cell line.
[So] Source:Exp Cell Res;352(2):393-402, 2017 Mar 15.
[Is] ISSN:1090-2422
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Cell lines established from tumors are the most commonly used models in cancer research, and their use in recent years has enabled a greater understanding of the biology of cancer and the means to develop effective treatment strategies. Astroblastomas are uncommon neuroepithelial tumors of glial origin, predominantly affecting young people, mainly teenagers and children, predominantly females. To date, only a single study has reported that astroblastomas contain a large number of neural stem-like cells, which had only a partial proliferation capacity and differentiation. Our objective was to establish an astroblastoma cell line to investigate the presence of astroblastic cells and cancer stem-like cells. The migratory and invasion abilities of the cells were quantified with invasion and migration assays and compared to a glioblastoma cell line. The presence of stem cells was detected with surface-marker analysis by using flow cytometry, and measuring the differentiation ability with a differentiation assay and the self-renewal capacity with a sphere-forming assay. These characteristics may determine whether this novel cell line is a model for astroblastomas that may have stem-cell characteristics. With this novel cell line, scientists can investigate the molecular pathways underlying astroblastomas and develop new therapeutic strategies for patients with these tumors.
[Mh] Termos MeSH primário: Técnicas de Cultura de Células/métodos
Neoplasias Neuroepiteliomatosas/patologia
Células-Tronco Neoplásicas/citologia
[Mh] Termos MeSH secundário: Linhagem Celular Tumoral
Separação Celular/métodos
Seres Humanos
Masculino
Células-Tronco Neoplásicas/fisiologia
Esferoides Celulares/citologia
Esferoides Celulares/fisiologia
Células Tumorais Cultivadas
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1705
[Cu] Atualização por classe:170526
[Lr] Data última revisão:
170526
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170225
[St] Status:MEDLINE



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